AB1852 DERMATO-NEURO SYNDROME PRECIPITATED BY COVID-19 INFECTION IN THE SETTING OF SCLEROMYXEDEMA
Authors: C. G. Hobayan, J. Lin
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> A black female in her 40s presented with a nonpruritic rash for 10 months consisting of bumps on the face, hands, forearms, and thighs. She had no prior treatment. Past medical history was significant for pulmonary embolism (PE) 6 years prior. She had no personal or family history of autoimmune disease. Physical exam revealed numerous smooth 2-3 mm skin-colored papules over the bilateral forearm dorsa, hands, anterior thighs, and face. Serum protein electrophoresis revealed monoclonal IgG lambda gammopathy. Skin biopsy of her left elbow showed dermal fibroplasia with mucin deposition. IgG was less than 1.5 grams/deciliter; bloodwork was otherwise stable. The diagnosis of scleromyxedema was rendered. <h3>Objectives</h3> The objective of this clinical case was to evaluate a neurologic sequela of COVID-19 infection in a patient with scleromyxedema. <h3>Methods</h3> One month following diagnosis of scleromyxedema, our patient was diagnosed with COVID-19 five days before admission to the emergency department with altered mental status and aphasia. Rheumatology was consulted due to malignant hypertension and acute kidney injury with question of scleroderma-like renal crisis in the setting of recently diagnosed COVID-19 infection, although she had no other features of systemic sclerosis. The infectious disease team was consulted due to COVID-19-induced inflammatory reaction. <h3>Results</h3> The patient’s creatinine kinase and brain natriuretic peptide were elevated. Creatinine and potassium trended upwards. She developed seizures and became hemodynamically unstable with rapidly declining clinical status. She was transferred to the intensive care unit, where she developed respiratory arrest, shock, hyperkalemia, and acidemia. She received escalating doses of pressors but experienced frequent arrhythmic disturbances and developed asystole. Resuscitation efforts were unsuccessful; she expired within 24 hours of consultation. <h3>Conclusion</h3> Dermato-neuro syndrome (DNS) is a potential complication of scleromyxedema associated with confusion, dysarthria, seizures, and coma. The patient’s clinical presentation is consistent with DNS in the setting of scleromyxedema likely precipitated by COVID-19. Intravenous immunoglobulins are first-line treatment for scleromyxedema; however, it is associated with risk of venous thromboembolism. The patient was considered for treatment as an outpatient but deferred due to history of PE. She was reevaluated for treatment upon presentation to the hospital, but given the severity and rapidity of her condition, it was already too late. This is the second reported case of COVID-19 induced DNS in a patient with scleromyxedema. Given the severity, we recommend early initiation of treatment in patients with scleromyxedema and aggressive treatment for those contracting COVID-19. <h3>References</h3> [1] Haber R, Bachour J, El Gemayel M. Scleromyxedema treatment: a systematic review and update. <i>Int J Dermatol</i>. 2020;59:1191-1201. [2] Flannery MT, Humphrey D. Deep Venous Thrombosis with Pulmonary Embolism Related to IVIg Treatment: A Case Report and Literature Review. <i>Case Rep Med</i>. 2015;971321. [3] Lee YH, Sahu J, O’Brien MS, D’Agati VD, Jimenez SA. Scleroderma Renal Crisis-Like Acute Renal Failure Associated With Mucopolysaccharide Accumulation in Renal Vessels in a Patient With Scleromyxedema. <i>J Clin Rheumatol</i>. 2011;17:318-322. [4] Hoffman-Vold AM, Distler O, Bruni C, et al. Systemic sclerosis in the time of COVID-19. <i>Lancet Rheumatol</i>. 2022;4:e566-575. [5] Fritz M, Tinker D, Wessel AW, et al. SARS-CoV-2: A potential trigger of dermato-neuro syndrome in a patient with scleromyxedema. <i>JAAD Case Rep</i>. 2021;18:99-102. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.